Fatal Acute Liver Failure in a Kidney-Transplant Recipient

36 Question: A 56-yearold man was transferred from another hospital with a rapid onset of jaundice, ascites and hepatic encephalopathy. He had a 7-year history of idiopathic hemolytic uremic syndrome leading to terminal renal failure, despite treatment with plasmapheresis and glucocorticoids. Cadaveric renal transplantation was performed 5 years previously. Immunosuppression consisted of tacrolimus, mycophenolate mofetil, and prednisolone. Serum tacrolimus trough levels ranged from 7 to 12 ng/mL. Pretransplant checkup including abdominal ultrasonography and liver function tests showed no abnormalities. About 7 months posttransplant, the patient developed anicteric chronic hepatitis with consistently elevated alanine aminotransferase (ALT) and aspartate aminotransferase levels ranging from 90 to 470 U/L, and 60 to 400 U/L, respectively (Figure A). Serologic tests for hepatitis B (HBV), C (HCV), E virus (HEV), Epstein–Barr virus, and cytomegalovirus, autoimmune serology (antinuclear antibody, anti-smooth muscle antibody, anti-mitochondrial antibody, and liver-kidney microsomal antibody-1) as well as HBV and HCV polymerase chain reaction (PCR) analyses from peripheral blood were repeatedly negative. Serum concentrations of a1 antitrypsin, copper, and ceruloplasmin were in the reference range. Liver biopsy showed distinct pericellular fibrosis with incomplete cirrhosis as well as minimal steatosis and mild inflammatory activity suggesting underlying steatohepatitis (Figure B). Iron and copper concentrations in the liver tissue were normal. Right ventricular failure, constrictive pericarditis, and Budd-Chiari syndrome were ruled out. Despite intensive therapy the patient lapsed into hepatic coma and died before liver transplantation could be performed. What is your diagnosis? Look on page 325 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.